DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20190314

Hereditary angioedema caused by C1-esterase inhibitor deficiency: a case report with literature-based analysis

Sufia Athar, Noureddine Korichi, Yousra Shehada Siam

Abstract


Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease caused by a mutation in the C1-inhibitor gene. It is a rare disease that is often worsened during pregnancy and childbirth. HAE, though uncommon but if untreated it may lead to maternal death.  The case report presents the successful management of a 24 years old, G2P1, with hereditary angioedema caused by C1-esterase inhibitor deficiency. This patient was managed with a multidisciplinary approach by an obstetrician, an immunologist, an anaesthesiologist and a pediatrician. She had an uneventful antenatal period, labor was induced. She had precipitate delivery and soon after delivery had a flare up of the disease. It was successfully managed with fresh frozen plasma and close observation.

 


Keywords


C1-esterase inhibitor deficiency (HAE-C1-INH), CI inhibitor concentrate, Fresh frozen plasma (FFP), Hereditary angioedema (HAE), Plasma derived human C1 inhibitor concentrate (pdhC1INH), Pregnancy

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